BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in people with cystic fibrosis (pwCF) can lead to severe outcomes. METHODS: In this observational study, the European Cystic Fibrosis Society Patient Registry collected data on pwCF and SARS-CoV-2 infection to estimate incidence, describe clinical presentation and investigate factors associated with severe outcomes using multivariable analysis. RESULTS: Up to December 31, 2020, 26 countries reported information on 828 pwCF and SARS-CoV-2 infection. Incidence was 17.2 per 1000 pwCF (95% CI: 16.0-18.4). Median age was 24 years, 48.4% were male and 9.4% had lung transplants. SARS-CoV-2 incidence was higher in lung-transplanted (28.6; 95% CI: 22.7-35.5) versus non-lung-transplanted pwCF (16.6; 95% CI: 15.4-17.8) (p≤0.001).SARS-CoV-2 infection caused symptomatic illness in 75.7%. Factors associated with symptomatic SARS-CoV-2 infection were age >40 years, at least one F508del mutation and pancreatic insufficiency.Overall, 23.7% of pwCF were admitted to hospital, 2.5% of those to intensive care, and regretfully 11 (1.4%) died. Hospitalisation, oxygen therapy, intensive care, respiratory support and death were 2- to 6-fold more frequent in lung-transplanted versus non-lung-transplanted pwCF.Factors associated with hospitalisation and oxygen therapy were lung transplantation, cystic fibrosis-related diabetes (CFRD), moderate or severe lung disease and azithromycin use (often considered a surrogate marker for Pseudomonas aeruginosa infection and poorer lung function). CONCLUSION: SARS-CoV-2 infection yielded high morbidity and hospitalisation in pwCF. PwCF with forced expiratory volume in 1 s <70% predicted, CFRD and those with lung transplants are at particular risk of more severe outcomes.

1 Tsitsishvili Children's Clinic CF Centre Tblisi Georgia

Adult Cystic Fibrosis Center Dept of Pulmonology Lithuanian University of Health Sciences Kaunas Lithuania

AP HP Hôpital Cochin Service de Physiologie et Explorations Fonctionnelles Paris France

Belarusian Republic Children's Center of Pulmonology and Cystic Fibrosis Pulmonary Department 3rd City Children's Clinical Hospital Minsk Belarus

Centre for Cystic Fibrosis Hospital de Santa Maria Lisbon Portugal

Centre for Cystic Fibrosis University Children's Hospital Skopje North Macedonia

Clinical Research Dept of Cystic Fibrosis Research Centre for Medical Genetics Moscow Russian Federation

Co first authors

Co senior authors

Cystic Fibrosis Centre University Hospital of Bratislava Bratislava Slovakia

Cystic Fibrosis Europe Brussels Belgium

Cystic Fibrosis Registry of Turkey Ankara Turkey

Cystic Fibrosis Trust London UK

Cystic Fibrosis Unit Hippokration General Hospital Aristotle University of Thessaloniki Thessaloniki Greece

Dept of Clinical Sciences and Community Health Laboratory of Medical Statistics Epidemiology and Biometry G A Maccacaro University of Milan Milan Italy

Dept of Medical Microbiology 2nd Faculty of Medicine Charles University and Motol University Hospital Prague Czech Republic

Dept of Paediatric Pulmonology University Children's Hospital Ljubljana University Medical Centre Ljubljana Slovenia

Dept of Paediatrics Cystic Fibrosis Regional Support Centre University of Brescia Brescia

Dept of Paediatrics Mother Thereza Hospital Center Tirana Albania

Dept of Paediatrics Norwegian Cystic Fibrosis Registry Oslo University Hospital Oslo Norway

Dept of Pathophysiology and Transplantation Cystic Fibrosis Reference Center of Lombardia Region Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico University of Milan Milan Italy

Dept of Pediatrics Ambulatory Cystic Fibrosis and Other Rare Diseases Center Institute for Maternal and Child Healthcare State University of Medicine and Pharmacy Nicolae Testemitanu Chisinau Republic of Moldova

Dept of Pediatrics and Adolescent Medicine Cystic Fibrosis Center Aarhus University Hospital Aarhus Denmark

Dept of Pediatrics and Adolescent Medicine Division of Pediatric Pulmonology and Allergology Medical University of Graz Graz Austria

Dept of Pneumology Children's Clinical University Hospital Rīga Stradinš University Riga Latvia

Dept of Pulmonology Hôpital Robert Schuman Luxembourg Luxembourg

Dept of Respiratory Paediatrics Royal Brompton Hospital London UK

Dutch Cystic Fibrosis Foundation Baarn The Netherlands

Dziekanow Paediatric Hospital Cystic Fibrosis Centre Institute of Mother and Child Warsaw Poland

European Cystic Fibrosis Society Karup Denmark

Institute of Hereditary Pathology Ukrainian National Academy of Medical Sciences Lviv Ukraine

Medical School University of Cyprus Nicosia Cyprus

National Centre for Cystic Fibrosis Mother and Child Health Institute of Serbia Dr Vukan Čupić Belgrade Serbia

NHLI Imperial College London UK

Paediatric Pulmonology University Children's Hospital Zurich Zurich Switzerland

Pediatric Clinic Alexandrovska University Hospital Medical University Sofia Bulgaria

Pediatric Pneumology and Cystic Fibrosis Unit Osakidetza Hospital Universitario Cruces Bizkaia Spain

Pediatric Pulmonology Dept and Cystic Fibrosis Center Sorbonne Université Centre de Recherche Saint Antoine Inserm UMR_S938 Assistance Publique Hôpitaux de Paris Hôpital Trousseau Paris France

Pulmonary Institute Schneider Children's Medical Center of Israel Petah Tikva Israel

Regional Respiratory Centre Belfast City Hospital Belfast UK

Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel

Sciensano Epidemiology and Public Health Health Services Research Brussels Belgium

Scientific Board of Italian CF Registry Rome Italy

St Vincent's University Hospital and University College Dublin School of Medicine Dublin Ireland

Stockholm CF Centre Karolinska University Hospital Karolinska Institutet Stockholm Sweden

Université Paris Descartes Sorbonne Paris Cité Paris France

Universities of Giessen and Marburg Lung Center German Center of Lung Research Justus Liebig University Giessen Giessen Germany

University Hospital Centre Zagreb Cystic Fibrosis Centre Paediatrics and Adults Zagreb Croatia

Victor Babes University of Medicine and Pharmacy Timisoara National Cystic Fibrosis Centre Timisoara Romania

Wellcome Wolfson Institute for Experimental Medicine Queen's University Belfast Belfast UK

Yerevan University CF Centre Muratsan Hospital Yerevan Armenia

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